Treatment-related Changes in Cerebrospinal Fluid Markers of Oxidative Stress and Neurodegeneration During Therapy for Childhood Acute Lymphoblastic Leukemia.

Background: Survivors of childhood acute lymphoblastic leukemia (ALL) frequently exhibit treatment-related neurocognitive impairment, although there is substantial interpatient variability in this outcome. Analysis of biomarkers that reflect the impact of chemotherapy during the two years of treatment for ALL offers the potential to identify children who have subclinical treatment-related neurotoxicity at a time when a protective intervention could prevent the development of persistent impairment.

Methods: We prospectively measured markers indicative of oxidative stress (8-hydroxydeoxyguanosine; 8OHdG) and neurodegeneration (total tau) in cerebrospinal fluid (CSF) collected at five timepoints before and during the first year of chemotherapy for ALL among 529 patients enrolled on DFCI ALL Consortium protocol 16-001 (NCT03020030).

Spontaneous retinal reattachment and novel vitreoretinal phenotype in muscle eye brain disease determined by fundus multimodal imaging.

IntroductionMuscle eye brain disease (MEB) is a rare, multi-systemic autosomal recessively inherited disorder of relevance to ophthalmologists. The aim of this report is to describe a novel ocular phenotype for a genetically confirmed MEB patient using retinal multi-modal imaging.Case descriptionWe report a case of 18-year-old male patient that was referred to our tertiary unit for management of retinal detachment.

HNF1A-inactived Hepatocellular Adenomas in Fibropolycystic Kidney and Liver Disease Are Associated with Germline HNF1B Variant.

HNF1A-inactivated hepatocellular adenomas are rarely described in patients with hepatorenal fibrocystic disease, and their genetic relationship remains unexplored. This study reports a series of HNF1A-inactivated hepatocellular adenomas with molecular sequencing analysis in patients with fibrocystic kidney and/or liver disease. Cases were retrospectively identified from the pathology archives of three institutions.

Neuroprotective Effects of a 3-Amino Quinazoline Derivative via Keap1-Nrf2 Pathway Activation in an ICV-STZ-Induced Rat Model of Sporadic Alzheimer's Disease.

The Keap1-Nrf2 pathway has emerged as a promising target for Alzheimer's disease (AD). This study employed modeling to identify Nrf2 activators through Keap1 inhibition. The most promising quinazoline derivative, LMDP10, was then evaluated in a rat model of sporadic AD induced by Intracerebroventricular (ICV) streptozotocin (STZ).

Objectively and Subjectively Measured Physical Activity and Their Associations With Cardiometabolic Risk in the UK Biobank: Retrospective Cohort Study.

Background: The association between physical activity (PA) behavior and cardiometabolic risk factors has depended largely on questionnaire-based reporting. More studies are turning to mobile health (mHealth) device solutions to measure PA. While there are differences between self-reported activity levels and objectively measured accelerometer-based activity, how these differences manifest in disease risk is unknown.